Congenital atrioventricular block is a rare, but severe occurrence in the newborn can be related to various cardiac malformations or to maternal autoimmune diseases, particularly systemic lupus erythematosus. We report an extremely low-birth-weight infant affected by congenital heart block, due to positive anti-RO/SSA and anti-LA/SSB antibodies of the mother, with progressively increasing respiratory distress syndrome and heart failure. The infant was successfully treated with an external constant-current, single-chamber ventricular (VVI) temporary epicardial pacing, placed on the second day of life, at the weight of 810 g. After this treatment, cardiac failure resolved and respiratory assistance could be rapidly stopped. A single-chamber, rate-responsive, permanent pacemaker was placed at 3 months of life with steroid-eluting, epicardial-pacing leads. We emphasize that early extrathoracic temporary epicardial pacing is able to resolve heart failure in an extremely low-birth-weight infant.